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March 01, 2016; 86 (9) Article

Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy

Jérôme J. Devaux, Yumako Miura, Yuki Fukami, Takayuki Inoue, Constance Manso, Maya Belghazi, Kenji Sekiguchi, Norito Kokubun, Hiroo Ichikawa, Anna Hiu Yi Wong, Nobuhiro Yuki
First published February 3, 2016, DOI: https://doi.org/10.1212/WNL.0000000000002418
Jérôme J. Devaux
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Yumako Miura
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Yuki Fukami
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Takayuki Inoue
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Constance Manso
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Maya Belghazi
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Kenji Sekiguchi
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Norito Kokubun
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Hiroo Ichikawa
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Anna Hiu Yi Wong
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Nobuhiro Yuki
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
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Citation
Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy
Jérôme J. Devaux, Yumako Miura, Yuki Fukami, Takayuki Inoue, Constance Manso, Maya Belghazi, Kenji Sekiguchi, Norito Kokubun, Hiroo Ichikawa, Anna Hiu Yi Wong, Nobuhiro Yuki
Neurology Mar 2016, 86 (9) 800-807; DOI: 10.1212/WNL.0000000000002418

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Abstract

Objective: We report the clinical and serologic features of Japanese patients with chronic inflammatory demyelinating polyneuropathy (CIDP) displaying anti-neurofascin-155 (NF155) immunoglobulin G4 (IgG4) antibodies.

Methods: In sera from 533 patients with CIDP, anti-NF155 IgG4 antibodies were detected by ELISA. Binding of IgG antibodies to central and peripheral nerves was tested.

Results: Anti-NF155 IgG4 antibodies were identified in 38 patients (7%) with CIDP, but not in disease controls or normal participants. These patients were younger at onset as compared to 100 anti-NF155–negative patients with CIDP. Twenty-eight patients (74%) presented with sensory ataxia, 16 (42%) showed tremor, 5 (13%) presented with cerebellar ataxia associated with nystagmus, 3 (8%) had demyelinating lesions in the CNS, and 20 of 25 (80%) had poor response to IV immunoglobulin. The clinical features of the antibody-positive patients were statistically more frequent as compared to negative patients with CIDP (n = 100). Anti-NF155 IgG antibodies targeted similarly central and peripheral paranodes.

Conclusion: Anti-NF155 IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset, ataxia, tremor, CNS demyelination, and a poor response to IV immunoglobulin. The autoantibodies may serve as a biomarker to improve patients' diagnosis and guide treatments.

GLOSSARY

Caspr1=
contactin-associated protein-1;
CCPD=
combined central and peripheral demyelination;
CI=
confidence interval;
CIDP=
chronic inflammatory demyelinating polyneuropathy;
CNTN1=
contactin 1;
GBS=
Guillain-Barré syndrome;
IgG4=
immunoglobulin G4;
IVIg=
IV immunoglobulin;
MS=
multiple sclerosis;
NF155=
neurofascin-155;
OR=
odds ratio;
PNS=
peripheral nervous system

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • ↵* These authors contributed equally to this work.

  • Supplemental data at Neurology.org

  • Editorial, page 796

  • Received May 7, 2015.
  • Accepted in final form October 1, 2015.
  • © 2016 American Academy of Neurology
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